Three cases of neonatal herpes simplex virus infection presenting as fulminant hepatitis.

We report three cases of neonatal herpes simplex virus (HSV) infection presenting as fulminant hepatitis. None of the patients had clear risk factors for HSV infection and they all died. Antiviral treatment for HSV is currently available but must be administered early in the course of the disease before irreversible liver tissue damage is present. Since the diagnosis may be difficult to establish, we wish to draw the attention of clinicians to the presentation of neonatal HSV infection and suggest that in such cases viral cultures, including culture of liver tissue, should be obtained early and antiviral treatment administered while awaiting the culture results.

Early-onset acquired epileptic aphasia (Landau-Kleffner syndrome, LKS) and regressive autistic disorders with epileptic EEG abnormalities: the continuing debate.

Early-onset acquired epileptic aphasia (Landau-Kleffner syndrome) may present as a developmental language disturbance and the affected child may also exhibit autistic features. Landau-Kleffner is now seen as the rare and severe end of a spectrum of cognitive-behavioural symptoms that can be seen in idiopathic (genetic) focal epilepsies of childhood, the benign end being the more frequent typical rolandic epilepsy. Several recent studies show that many children with rolandic epilepsy have minor developmental cognitive and behavioural problems and that some undergo a deterioration (usually temporary) in these domains, the so-called "atypical" forms of the syndrome. The severity and type of deterioration correlate with the site and spread of the epileptic spikes recorded on the electroencephalogram within the perisylvian region, and continuous spike-waves during sleep (CSWS) frequently occur during this period of the epileptic disorder. Some of these children have more severe preexisting communicative and language developmental disorders. If early stagnation or regression occurs in these domains, it presumably reflects epileptic activity in networks outside the perisylvian area, i.e. those involved in social cognition and emotions. Longitudinal studies will be necessary to find out if and how much the bioelectrical abnormalities play a causal role in these subgroup of children with both various degrees of language and autistic regression and features of idiopathic focal epilepsy. One has to remember that it took nearly 40 years to fully acknowledge the epileptic origin of aphasia in Landau-Kleffner syndrome and the milder acquired cognitive problems in rolandic epilepsies.

Identification of an RP1 Prevalent Founder Mutation and Related Phenotype in Spanish Patients with Early-Onset Autosomal Recessive Retinitis.

OBJECTIVE: To identify the genetic causes underlying early-onset autosomal recessive retinitis pigmentosa (arRP) in the Spanish population and describe the associated phenotype. DESIGN: Case series. PARTICIPANTS: A total of 244 unrelated families affected by early-onset arRP. METHODS: Homozygosity mapping or exome sequencing analysis was performed in 3 families segregating arRP. A mutational screening was performed in 241 additional unrelated families for the p.Ser452Stop mutation. Haplotype analysis also was conducted. Individuals who were homozygotes, double heterozygotes, or carriers of mutations in RP1 underwent an ophthalmic evaluation to establish a genotype-phenotype correlation. MAIN OUTCOME MEASURES: DNA sequence variants, homozygous regions, haplotypes, best-corrected visual acuity, visual field assessments, electroretinogram responses, and optical coherence tomography images. RESULTS: Four novel mutations in RP1 were identified. The new mutation p.Ser542Stop was present in 11 of 244 (4.5%) of the studied families. All chromosomes harboring this mutation shared the same haplotype. All patients presented a common phenotype with an early age of onset and a prompt macular degeneration, whereas the heterozygote carriers did not show any signs of retinitis pigmentosa (RP). CONCLUSIONS: p.Ser542Stop is a single founder mutation and the most prevalent described mutation in the Spanish population. It causes early-onset RP with a rapid macular degeneration and is responsible for 4.5% of all cases. Our data suggest that the implication of RP1 in arRP may be underestimated. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Conhecimento e percepção dos profissionais a respeito do ruído na unidade neonatal

O objetivo deste estudo foi verificar o conhecimento e a percepção de profissionais de Unidade de Terapia Intensiva Neonatal (UTIN) sobre as repercussões do ruído ao neonato, família e profissionais, antes da implementação de um programa educativo. Trata-se de uma pesquisa descritiva, quantitativa, realizada em Unidade de Terapia Intensiva Neonatal de um hospital de São Paulo, com 101 profissionais. Foram utilizados: questionário para a coleta de dados, e os testes de Qui-quadrado e t de Student para a associação entre as variáveis. Os profissionais identificaram a UTIN como muito ruidosa (44,9%), perceberam os efeitos desse ruído durante e após a jornada de trabalho (67,4%) e utilizaram estratégias para amenizá-lo. Embora os profissionais desconheçam a legislação sobre o ruído no ambiente hospitalar, identificaram repercussões para si, recém-nascido e família. Os resultados apontaram para a necessidade de orientar a equipe quanto à legislação, prevenção de ruído e reorganização das práticas assistenciais e estrutura física da Unidade de Terapia Intensiva Neonatal.

Mortalidade pós-neonatal no território brasileiro: uma revisão da literatura

O presente trabalho trata-se de revisão sistemática, referente ao período de 2004 a 2009, sobre o tema mortalidade pós-neonatal. Teve o objetivo de identificar como se colocam na literatura, as causas da morte e a relação com as condições socioeconômicas. Foram selecionados 27 artigos, 74,4% publicados em periódicos da área da Saúde Pública e 66,7%, de desenho do tipo ecológico. Quase a totalidade versava sobre grupos de causas e seus componentes (66,7%), seguidos pelo terço restante, sobre a identificação dos fatores determinantes dos óbitos. A região Sudeste produziu mais de 37% dos estudos. Na maioria dos municípios e estados brasileiros, a redução superou 50% no final da década de 1990. Dentre os grupos de causas de óbitos, predominou o grupamento diarreia-pneumonia, seguido pelas malformações congênitas. As condições de vida segundo indicadores socioeconômicos - moradia, saneamento básico, educação e acesso à saúde - foram determinantes para os maiores índices de mortalidade pós-neonatal por causas passíveis de redução.

Effect of obesity onset on pendular energy transduction at spontaneous walking speed: Prader-Willi versus nonsyndromal obese individuals.

OBJECTIVE: To compare the mechanical external work (Wext ) and pendular energy transduction (Rstep ) at spontaneous walking speed (Ss ) in individuals with Prader-Willi syndrome (PWS) versus subjects with nonsyndromal obesity (OB) to investigate whether the early onset of obesity allows PWS subjects to adopt energy conserving gait mechanics. DESIGN AND METHODS: Wext and Rstep were computed using kinematic data acquired by an optoelectronic system and compared in 15 PWS (BMI = 39.5 ± 1.8 kg m(-2) ; 26.7 ± 1.5 year) and 15 OB (BMI = 39.3 ± 1.0 kg m(-2) ; 28.7 ± 1.9 year) adults matched for gender, age and BMI and walking at Ss . RESULTS: Ss was significantly lower in PWS (0.98 ± 0.03 m s(-1) ) than in OB (1.20 ± 0.02 m s(-1) ; P < 0.001). There were no significant differences in Wext per kilogram between groups (PWS: 0.37 ± 0.04 J kg(-1) m(-1) ; OB: 0.40 ± 0.05 J kg(-1) m(-1) ; P = 0.66) and in Rstep (PWS: 69.9 ± 2.9%; OB: 67.7 ± 2.4%; P = 0.56). However, Rstep normalized to Froude number (Rstep /Fr) was significantly greater in PWS (6.0 ± 0.6) than in OB (3.8 ± 0.2; P = 0.001). Moreover, Rstep /Fr was inversely correlated with age of obesity onset (r = -0.49; P = 0.006) and positively correlated with obesity duration (r = 0.38; P = 0.036). CONCLUSION: Individuals with PWS seem to alter their gait to improve pendular energy transduction as a result of precocious and chronic adaptation to loading.

Estradiol and progesterone strongly inhibit the innate immune response of mononuclear cells in newborns.

Newborns are particularly susceptible to bacterial infections due to qualitative and quantitative deficiencies of the neonatal innate immune system. However, the mechanisms underlying these deficiencies are poorly understood. Given that fetuses are exposed to high concentrations of estradiol and progesterone during gestation and at time of delivery, we analyzed the effects of these hormones on the response of neonatal innate immune cells to endotoxin, bacterial lipopeptide, and Escherichia coli and group B Streptococcus, the two most common causes of early-onset neonatal sepsis. Here we show that at concentrations present in umbilical cord blood, estradiol and progesterone are as powerful as hydrocortisone for inhibition of cytokine production by cord blood mononuclear cells (CBMCs) and newborn monocytes. Interestingly, CBMCs and newborn monocytes are more sensitive to the effects of estradiol and progesterone than adult peripheral blood mononuclear cells and monocytes. This increased sensitivity is associated with higher expression levels of estrogen and membrane progesterone receptors but is independent of a downregulation of Toll-like receptor 2 (TLR2), TLR4, and myeloid differentiation primary response gene 88 in newborn cells. Estradiol and progesterone mediate their anti-inflammatory activity through inhibition of the NF-κB pathway but not the mitogen-activated protein kinase pathway in CBMCs. Altogether, these results suggest that elevated umbilical cord blood concentrations of estradiol and progesterone acting on mononuclear cells expressing high levels of steroid receptors contribute to impair innate immune responses in newborns. Therefore, intrauterine exposure to estradiol and progesterone may participate in increasing susceptibility to infection during the neonatal period.

Fatores determinantes da mortalidade neonatal em um município da Região Sul do Brasil

Pesquisa quantitativa do tipo ecológico cujo objetivo foi identificar os fatores de risco que determinaram o óbito neonatal no município de Londrina, Paraná, entre 2000 e 2009. Verificou-se que idade materna, escolaridade, renda familiar, ocupação, situação conjugal, tipo de parto e número de consultas pré-natais não se associaram ao óbito neonatal. Entretanto, o peso ao nascer, a idade gestacional, o índice de Apgar no 1º e 5º minutos e local do parto mostraram-se estatisticamente significativos. Mais de 73,0% dos recém-nascidos evoluíram para óbito no período neonatal precoce. A causa básica predominante foi a afecção perinatal (77,7%), sendo que 72,6% das mortes foram consideradas evitáveis e a maioria, reduzível por controle adequado da gravidez e do parto. Tais resultados reforçam a necessidade de investimentos na prevenção do parto prematuro com assistência antenatal e ao parto equitativa, acessível e integral entre os diferentes níveis de atenção à saúde materno-infantil.

Fatores associados à infecção pelo uso do cateter central de inserção periférica em Unidade de Terapia Intensiva Neonatal

Estudo epidemiológico, longitudinal e analítico, desenvolvido em um hospital de Minas Gerais, com o objetivo de analisar os fatores associados à infecção pelo uso do cateter central de inserção periférica em recém-nascidos internados em unidade de terapia intensiva. A coleta dos dados foi realizada por meio de uma ficha estruturada, preenchida pelos profissionais e verificada pelos pesquisadores. Foram estudados 291 cateteres inseridos em 233 recém-nascidos. Os fatores associados à retirada por suspeita de infecção foram: prematuridade, peso ao nascer até 1.500 gramas, cateter de poliuretano, localização não centralizada do cateter e tempo de uso superior a 30 dias. Após ajuste multivariado, permaneceram independentemente associados: peso inferior a 2.500 gramas na inserção, reparo e tempo de uso do cateter. Conclui-se que fatores relacionados à prática dos profissionais contribuíram para a retirada dos cateteres, sinalizando para a necessidade de intervenções que melhorem a segurança e a eficácia em seu uso.

O processo do cateterismo venoso central em Unidade de Terapia Intensiva Neonatal e Pediátrica

Estudo descritivo, longitudinal e abordagem quantitativa, que objetivou analisar e discutir o processo do cateterismo venoso central nas Unidades de Terapia Intensiva neonatal e pediátrica; descrever as variáveis relacionadas à caracterização da população do estudo (unidade de internação, faixa etária e sexo) e descrever as variáveis relacionadas ao processo do cateterismo venoso central (tipo de cateter, motivo de indicação, número de lumens, sítio de inserção, profissional que realizou o procedimento, terapêutica medicamentosa infundida via cateter, motivo de retirada, tempo de permanência e as complicações mecânicas e infecciosas). A coleta de dados foi realizada em unidade de terapia intensiva neonatal e pediátrica, em 82 prontuários. As indicações dos cateteres foram, em sua maioria, para infusão medicamentosa prolongada e Nutrição Parenteral Total. A remoção foi indicada predominantemente por complicações mecânicas e infecciosas. Esse estudo viabilizou rever a prática assistencial para estabelecer o aprimoramento da assistência prestada à clientela neonatal e pediátrica.

Human milk for neonatal pain relief during ophthalmoscopy

Ophthalmoscopy performed for the early diagnosis of retinopathy of prematurity (ROP) is painful for preterm infants, thus necessitating interventions for minimizing pain. The present study aimed to establish the effectiveness of human milk, compared with sucrose, for pain relief in premature infants subjected to ophthalmoscopy for the early diagnosis of ROP. This investigation was a pilot, quasi-experimental study conducted with 14 premature infants admitted to the neonatal intensive care unit (NICU) of a university hospital. Comparison between the groups did not yield a statistically significant difference relative to the crying time, salivary cortisol, or heart rate (HR). Human milk appears to be as effective as sucrose in relieving acute pain associated with ophthalmoscopy. The study’s limitations included its small sample size and lack of randomization. Experimental investigations with greater sample power should be performed to reinforce the evidence found in the present study.

A manipulacao de prematuros em uma Unidade de Terapia Intensiva Neonatal

Os prematuros são submetidos a diversas manipulações e procedimentos durante internação em unidades neonatais, com consequências deletérias para a saúde. O objetivo do presente estudo foi descrever a manipulação a que são submetidos os prematuros durante as 24 horas em uma unidade de terapia intensiva neonatal (UTIN). Estudo observacional, descritivo, exploratório, realizado com 20 prematuros, filmados continuamente por um período de 24 horas, de setembro de 2008 a março de 2009 numa UTIN. Os prematuros foram submetidos a uma média de 768 manipulações e 1.341 procedimentos. A manipulação durou em média 2 horas e 26 minutos em um período de 24 horas. Cada manipulação agrupou uma média de 2,2 procedimentos, a maioria no turno matutino As manipulações isoladas representaram 65,6% do total de manipulações e a maioria teve duração inferior a um minuto. Conclui-se que nas 24 horas avaliadas, os prematuros foram submetidos a um excesso de manipulações na UTIN.

Intimate Partner Violence Against Pregnant Women: Study About The Repercussions On The Obstetric And Neonatal Results

This observational, descriptive and analytic study aimed to identify the prevalence of IPV cases among pregnant women and classify them according to the type and frequency; identify the obstetric and neonatal results and their associations with the intimate partner violence (IPV) occurrence in the current pregnancy. It was developed with 232 pregnant women who had prenatal care at a public maternity hospital. Data were collected via structured interview and in the patients’ charts and analyzed through the statistic software SAS® 9.0. Among the participants, 15.5% suffered IPV during pregnancy, among that 14.7% suffered psychological violence, 5.2% physical violence and 0.4% sexual violence. Women who did not desire the pregnancy had more chances of suffering IPV (p<0.00; OR=4.32 and 95% CI [1.77 – 10.54]). With regards to the obstetric and neonatal repercussions, there was no statistical association between the variables investigated. Thus, for the study participants there were no negative obstetric and neonatal repercussions related to IPV during pregnancy.

Neutrophils contribute to development of a protective immune response during onset of infection with Leishmania donovani.

Neutrophils are key components of the inflammatory response and as such contribute to the killing of microorganisms. In addition, recent evidence suggests their involvement in the development of the immune response. The role of neutrophils during the first weeks post-infection with Leishmania donovani was investigated in this study. When L. donovani-infected mice were selectively depleted of neutrophils with the NIMP-R14 monoclonal antibody, a significant increase in parasite numbers was observed in the spleen and bone marrow and to a lesser extent in the liver. Increased susceptibility was associated with enhanced splenomegally, a delay in the maturation of hepatic granulomas, and a decrease in inducible nitric oxide synthase expression within granulomas. In the spleen, neutrophil depletion was associated with a significant increase in interleukin 4 (IL-4) and IL-10 levels and reduced gamma interferon secretion by CD4(+) and CD8(+) T cells. Increased production of serum IL-4 and IL-10 and higher levels of Leishmania-specific immunoglobulin G1 (IgG1) versus IgG2a revealed the preferential induction of Th2 responses in neutrophil-depleted mice. Altogether, these data suggest a critical role for neutrophils in the early protective response against L. donovani, both as effector cells involved in the killing of the parasites and as significant players influencing the development of a protective Th1 immune response.

Neonatal mortality in infants with low birth weigh

Objective To evaluate the factors associated with neonatal mortality in infant born with low birth weight. Method Cross-sectional study that analyzed data from 771 live births with low birth weight (<2500 g) in the city of Cuiabá, MT, in 2010, of whom 54 died in the neonatal period. We obtained data from the Information System on Live Births and Mortality, by integrated linkage. Results In multiple logistic regression, neonatal mortality was associated with: number of prenatal visits less than 7 (OR=3.80;CI:1,66-8,70); gestational age less than 37 weeks (OR=4.77;CI:1.48-15.38), Apgar score less than 7 at the 1st minute (OR=4.25;CI:1.84-9.81) and the 5th minute (OR=5.72,CI:2.24-14.60) and presence of congenital anomaly (OR=14.39;IC:2.72-76.09). Conclusion Neonatal mortality in infants with low birth weight is associated with avoidable factors through adequate attention to prenatal care, childbirth and infants.